Step 3 Ch 7 Hematology 2-0

Front 1

201. Platelet count in von Willebrand's disease?

Back 1

a. The platelet count is normal.

Front 2

202. aPTT in von Willebrand's?

Back 2

a. Can be elevated in up to 50% of patients with VWD, because VWF deficiency destabilizes factor VIII.

Front 3

203. Most accurate diagnostic test for von Willebrand's disease?

Back 3

a. Ristocetin cofactor assay
b. and
c. von Willebrand factor (VWF) level.
d. If the level of VWF is normal, ristocetin testing will tell if it is working properly.

Front 4

204. Treatment of von Willebrand's disease?

Back 4

a. Desmopressin or DDAVP.
1. This will release sub-endothelial stores of VWF and factor VIII which will help stop the bleeding.
2. If Desmopressin is not effective, the next best step in management is to use factor VIII replacement.
3. Factor VIII replacement has both VWF and factor VIII.

Front 5

205. Note: a case of VW D is likely to present with epistaxis and/or petechiae.

Back 5

Note: a case of VW D is likely to present with epistaxis and/or petechiae.

Front 6

206. Presentation of idiopathic thrombocytopenic purpura (ITP)?

Back 6

a. ITP presents with platelet type bleeding if the platelet count is <50,000.

Front 7

207. diagnostic testing for ITP?

Back 7

1. Antiplatelet antibodies
2. sonogram: to assess for normal spleen size found in ITP
3. bone marrow: to find increased numbers of megakaryocytes.
4. Antibodies to glycoprotein 2B/3A receptor.

Front 8

208. Qualities of platelet-type bleeding?

Back 8

1. Petechiae
2. Epistaxis
3. Purpura
4. gingiva
5. gums
6. vaginal

Front 9

209. Qualities of factor type bleeding?

Back 9

a. Hemarthrosis
b. Hematoma.

Front 10

210. Generally healthy patient comes epistaxis and petechiae. No spleen is felt on examination. The platelet count is 24,000. What is the next step in management?

Back 10

a. Answer: A- Prednisone
1. prednisone is the most important thing to do in mild ITP.
2. The main point of most ITP questions is that initiating therapy is more important than determining a specific diagnosis, particularly since ITP is a diagnosis of exclusion.out

Front 11

211. the patient comes in with ITP and a platelet count of 5000. the patient has epistaxis and petechiae, as well as an intracranial hemorrhage and melena. What is the best initial step?

Back 11

a. Answer: IVIG administration.
b. The fastest way to raise the plan account with ITP is to use intravenous immunoglobulins or RhoGAM.
c. IVIG is the answer when the platelet count is low (<20,000) in the case describes life-threatening bleeding, such as that into the bowel or brain.
d. Transfusion platelets is wrong; this adds fuel to the fire and will likely make the situation worse.

Front 12

212. Treatment of chronic ITP ( two drugs)?

Back 12

1. Romiplostim
2. Eltromopag
b. MOA of Romiplostim and Eltromopag?
1. They directly stimulate megakaryocytes.

Front 13

213. Case presents with platelet count > 50,000. Treatment?

Back 13

a. No treatment

Front 14

214. Case presents with platelet count < 50,000 with minor bleeding. Treatment?

Back 14

a. prednisone

Front 15

215. Case presents with platelet count <20,000 with serious bleeding . Treatment?

Back 15

a. IVIG or Rhogam (Rho[D] noon globulin)

Front 16

216. Case presents with recurrent episodes of ITP. Treatment?

Back 16

a. splenectomy

Front 17

217. Case presents with no response to splenectomy . Treatment?

Back 17

a. Romiplostim or
b. Eltrombopag.

Front 18

218. Pathophysiology of uremia-induced platelet dysfunction?

Back 18

a. Uremia by itself prevents platelets from working properly.
b. They do not deregulate!!!

Front 19

219. presentation of uremia-induced platelet dysfunction?

Back 19

a. Look for a normal platelet count with platelet-type bleeding in a patient with renal failure.
b. The ristocetintest and VWF level will be normal.

Front 20

220. Best initial therapy for uremia-induced platelet dysfunction?

Back 20

a. Desmonpressin (DDAVP)